Discussion

Extra-adrenal pheochromocytoma is an uncommon manifestation of a rare disease, and historical series have reported open surgical techniques to achieve cure. As biochemical testing and radiographic imaging have improved, minimally invasive procedures have been introduced into the management of these patients.

The advent of laparoscopy, combined with the development of sensitive biochemical assays and improved imaging, required reevaluation of all the aspects of the management of pheochromocytoma, including extra-adrenal tumors.

Review of the literature reveals a limited number of series evaluating results of laparoscopic management of extra-adrenal pheochromocytoma (20-22). Table 1 summarizes the data from those series.

Size of tumor has not been a contraindication to a minimally invasive approach, as our last patient had a 6.7 cm tumor successfully resected. Review of laparoscopic series shows that of 24 paragangliomas resected in 17 patients, 23/24 were = 4 cm in size. Sixteen of these 17 patients underwent successful laparoscopic or retroperitoneo-scopic resection of tumors. One conversion in our series occurred in a patient who had significant adhesion between the paraganglioma, the aorta, and the renal hilum. Postoperative complications are infrequent and are related to pulmonary and positional issues, with one patient from the National Cancer Institute series developing a gluteal hematoma and lower-extremity lymph edema which resolved with conservative management. Noteworthily, one patient in Janetscek's series developed self-limiting lymph ascites, which resolved after four weeks (22).

Vigilant monitoring and control of intra-operative blood pressure are critical in patients with pheochromocytoma. Intraoperative hypertensive crisis can be avoided with adequate preoperative blockade.

The National Cancer Institute series had only one of seven patients with a systolic blood pressure greater than 200 mmHg, whereas Walz had only 4 of 52 patients undergoing laparoscopic surgery for adrenal or extra-adrenal pheochromocytoma with a systolic blood pressure greater than 200 mmHg (20,21).

Paragangliomas have been linked to more aggressive pathologic behaviors with malignancy rates up to 50% in some series (15,23,24). Neither Walz nor Janetschek reported any evidence of local recurrence in their respective series, although noted a

TABLE 1 â–  Review of Laparoscopic Extra-Adrenal Literature

Concurrent

Operating

Estimated

Multiple

Surgical

adrenalectomy

Tumor

room time

blood loss

paraganglioma

No.

Series

n

Hereditary

approach

(no. of patients)

size (cm)

(min)

(cc)

(no. of patients)

converted

NCI-Walther

7

4

Transperitoneal

1-Unilateral

1.8-6.7

296

314

1

1

1-Bilateral

Janetschek

4

4

Transperitoneal

4-Bilateral

2.0-3.5

390-600

100-340

2

0

Walz

e

2

4-Transperitoneal

2-Unilateral

1.0-4.0

75-600

NR

3

0

2-Retroperitoneal

Abbreviations: NCI, National Cancer Institute; NR, Not recorded. Source: From Refs. 20-22.

The advent of laparoscopy, combined with the development of sensitive biochemical assays and improved imaging, required reevaluation of all the aspects of the management of pheochromocytoma, including extra-adrenal tumors.

Vigilant monitoring and control of intraoperative blood pressure are critical in patients with pheochromocytoma. Intraoperative hypertensive crisis can be avoided with adequate preoperative blockade.

short follow-up interval. In their series, with a follow range of 6 to 48 months, the authors have not detected any local or distant recurrences.

Pheochromocytomas of the bladder are rare, with mostly case reports in the literature. Management of these tumors should parallel the approach to extra-adrenal pheochromocytoma.

Once the tumor is localized, adequate preoperative blockade is performed prior to any manipulation. Cystoscopically, these tumors tend to be submucosal with occasional mucosal ulceration (10,25-27).

Kozlowski et al. reported a case of successful laparoscopic partial cystectomy for extra-adrenal pheochromocytoma using a combination of endoscopic and laparo-scopic excision (25). Endoscopically, a Collin's knife was used to excise the intravesical component while a standard four-diamond laparoscopic port placement, using ultrasonic shears, was used to complete the extravesical mobilization and excision of the tumor. A laparoscopic sac was used to remove the specimen. The defect was then repaired using interrupted 2-0 polyglactin sutures placed free hand and with an EndoStitch®b device (25).

Transurethral resection bladder tumor has been utilized in select case reports (10,27). However, partial cystectomy should be the initial approach to treat pheochromocytoma of the bladder. Although laparoscopic cystectomy and partial cystectomy are still in their infancy, they are a viable approach to all tumors of the bladder.

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